Recurrent Pulmonary Edema in a patient with bilateral renal artery stenosis:

A Case report on Pickering Syndrome

Dewasish S. Swar1, Anju Paudel1*, Suman Khanal2, Saurav Jha1, Prabhat K.C1

1. Department of Medicine, Patan Academy of Health Sciences

2. Department of Medicine, Nepalgunj Medical College Teaching Hospital

Author information:

1. Dewasish Singh Swar (Primary author)

Patan Academy of Health Sciences
Email address: dewasish.swar@gmail.com
Orchid ID: 0009-0005-3187-9032

2. Anju Paudel (Corresponding author)

Patan Academy of Health Sciences
Email address: dranjupaudel@gmail.com
Orchid ID: 0009-0008-7564-4616

3. Suman Khanal
Nepalgunj Medical College Teaching Hospital
Email address: skhanal28.stidh@gmail.com
Orchid ID: 0009-0003-2698-3033

4. Saurav Jha
Patan Academy of Health Sciences
Email address: sauravjha568@gmail.com
Orchid ID: 0000-0001-7967-0039

5. Prabhat K.C
Patan Academy of Health Sciences
Email address: prabhatkc@pahs.edu.np1
Orchid ID: 0000-0002-7356-6684

1
Corresponding author: Dr. Anju Paudel
Email address: dranjupaudel@gmail.com
KEY CLINICAL MESSAGE

Pickering syndrome should be strongly suspected in patients with refractory hypertension,

recurrent flash pulmonary edema, and progressive CKD. Diagnosing renal artery stenosis early
with appropriate imaging, such as renal Doppler ultrasound is crucial. Timely recognition and
revascularization may help prevent fatal complications.

ABSTRACT

Pickering syndrome is a rare clinical entity characterized by recurrent episodes of flash

pulmonary edema (FPE) and systemic hypertension secondary to bilateral renal artery stenosis
(RAS) or unilateral stenosis in a solitary functioning kidney. We present the case of a 43-year-old
male with chronic kidney disease (CKD) and poorly controlled hypertension who manifested
with hypertensive emergency and multiple episodes of FPE. Subsequent diagnostic evaluation
revealed critical bilateral RAS. The patient underwent right renal artery stenting; however, he
experienced fatal hyperkalemic cardiac arrest a few days after the procedure. This case
underscores the imperative to clinically suspect RAS in patients with refractory hypertension,
recurrent FPE and progressive CKD. Early identification and prompt revascularization may
mitigate adverse outcomes in high-risk population.

Keywords: Renal artery stenosis, Pulmonary edema, Renal revascularization, Chronic Kidney
Disease, Pickering Syndrome

INTRODUCTION

Pickering Syndrome is recurrent flash pulmonary edema affecting patients with hypertension and
bilateral renal artery stenosis (RAS) or significant unilateral stenosis in a single functioning
kidney and impaired renal function.1 Flash pulmonary edema (FPE) is a general term used to
describe a hyper-acute presentation of decompensated heart failure due to an acute rise in left
ventricular end diastolic pressure.2 However, Pickering Syndrome differs from the usual causes
of left ventricular failure with pulmonary edema as this condition is not associated with left
ventricular systolic dysfunction.3
We report the case of a 43-year-old male with bilateral renal artery stenosis who developed
recurrent episodes of severe pulmonary edema requiring mechanical ventilation in the
background of resistant hypertension and CKD. The patient was scheduled for bilateral renal
artery stenting but died 2 days after right renal artery stenting. Pulmonary edema is a common
presentation among patients in the Emergency Department. In addition to cardiac causes, renal
artery stenosis can also be a cause that may be missed in our setting.

CASE REPORT

Case History/Examination

A 43-year-old male, a known case of stage V CKD for 10 months and hypertension for 3 years
presented with dyspnea, chest heaviness, confusion and drowsiness for one week. He was not on
hemodialysis. His blood pressure at presentation was 220/160 mmHg in right arm and 220/170
mmHg in the left arm.

His medical history included HTN poorly managed with four antihypertensives agents and a
previous hospital admission for hypertensive urgency with hypertensive retinopathy. CKD stage
V with residual kidney function of approximately 1500-2000 ml of urine per day. He also
reported experiencing 2-3 episodes of sudden onset shortness of breath at home in the last 5-6
months that resolved spontaneously within hours. Notably, most episodes occurred during night.

Investigations, diagnosis and treatment

His initial lab reports showed elevated Urea (259mg/dl), Creatinine (18.7md/dl) and K(5.6meq/l)
with eGFR of 3ml/min/1.73m2. Urinalysis revealed 3+ albuminuria, and the hemoglobin was
6.6gm/dl consistent with anemia of chronic disease. ABG showed metabolic acidosis with
respiratory compensation.

In view of hypertensive encephalopathy and refractory hyperkalemia, the patient was scheduled
for Emergency Hemodialysis. After 20 minutes of hemodialysis, he developed sudden onset
respiratory distress with oxygen saturation dropping to 45% on room air and GCS score of 8/15.
Chest auscultations revealed bilateral diffuse crackles and occasional wheezes necessitating
immediate intubation and mechanical ventilation. The lung’s point of care ultrasound (POCUS)
showed multiple B lines throughout both lung fields. Based on the appearance of B lines, acute
pulmonary edema was suspected, and a chest radiograph (Figure 1) supported the diagnosis.
Echocardiography revealed Concentrated LVH, Normal heart chambers, grade 1 LVDD with
raised LVEDP (E/e’=20), Normal LVEF = 57%, and minimal pericardial effusion.

The patient was started on furosemide infusion at 2mg/min, which was gradually increased to
10mg/min and nitroglycerine infusion at 5mcg/minute was titrated up to 200mcg/min. The
patient showed a poor clinical response, eventually requiring dialysis for resolution of
Pulmonary edema. The patient was extubated 3 days after intubation.
Outcome

Throughout the hospital stay the patient had persistent uncontrolled hypertension requiring
continuous labetalol infusion, a gradual decrease in urine output and refractory hyperkalemia
requiring dialysis every alternate day. A total of 12 sessions were performed during his 25-day
hospital stay. There was a total of 3 episodes of sudden respiratory deterioration requiring
intubation and mechanical ventilation. POCUS and chest radiograph findings were suggestive of
Pulmonary Edema each time.

The patient’s multiple episodes of flash pulmonary edema, combined with resistant hypertension
and progressive renal dysfunction led to suspicion of renovascular cause. Bilateral renal artery
Doppler revealed high-resistance flow, with the right main renal artery showing a peak systolic
velocity (PSV) of 173 cm/s and a resistive index of 0.9. In comparison, the left main renal artery
demonstrated a PSV of 145 cm/s and a resistive index of 0.1 as illustrated in Figure 2.
Significantly reduced flow in the segmental arteries was also observed, supporting the findings
of bilateral renal artery stenosis. A bilateral renal angiogram confirmed stenosis at the origins of
both renal arteries (Figure 3 and 4) and the patient underwent successful stenting of the right
renal artery. However, the patient developed worsening renal function test for next two days after
procedure leading to death due to hyperkalemic cardiac arrest.

DISCUSSION

In a Lancet article in 1988, Pickering et al. were the first to describe the association between
acute recurrent pulmonary edema and renal artery stenosis. 5 Most patients with FPE have
bilateral RAS however in both present studies and Pickering series it was also seen in few
patients with unilateral RAS.5,6 Atherosclerosis and fibromuscular dysplasia are the most
common causes of RAS with atherosclerosis accounting for more than 90% of RAS lesions.
Atherosclerotic disease commonly involves the ostium and the proximal third of the main renal
artery.7

FPE is a clinical term used to describe a dramatic and life-threatening form of acute
decompensated heart failure.6 Bilateral renal artery stenosis (RAS) predisposes patients to flash
pulmonary edema (FPO) through three main mechanisms: defective natriuresis, increased
hemodynamic burden with diastolic dysfunction, and pulmonary capillary barrier failure. Unlike
unilateral RAS, in which the contralateral kidney compensates for sodium excretion and suppress
renin, bilateral RAS prevents this compensation, leading to persistent sodium and fluid retention.
Sustained hypertension due to RAS contributes to left ventricular hypertrophy (LVH) and
diastolic dysfunction, leading to elevated pulmonary venous pressure. Additionally, systemic
vascular resistance increases the myocardial oxygen demand and worsens LV function.
Pulmonary capillary stress failure occurs when high pressure forces into alveolar spaces.
Neurohumoral mediators such as angiotensin II and endothelin-1 further increase capillary
permeability. In decompensated bilateral RAS, small fluctuations in blood pressure or renal
perfusion can trigger a vicious cycle of worsening cardiac and renal function, perpetuating FPO
episodes.4

It is interesting to note that, in many studies, patients experienced FPE only at night. It is
suggested that these patients might have severe renal artery lesions that are noncritical during the
day with normal ambulatory blood pressure but with a nocturnal dip in blood pressure, the lesion
becomes critical provoking severe perturbation of the renin angiotensin system.8

Most patients with Pickering syndrome are underdiagnosed. An important factor contributing to
overlooking Pickering syndrome could be the risk associated with using contrast for imaging the
renal arteries which could worsen CKD in patients with already impaired eGFR. 1 We used Renal
artery doppler as a primary tool for diagnosing RAS in our case. Doppler assessment of renal
artery flow velocity and intrarenal resistivity indices (RIs) is an important diagnostic and
screening tool with a sensitivity of up to 90% and specificity of up to 95% for diagnosing
significant RAS. 1,2

Prior to the availability of angioplasty, surgical repair was the only option for the correction of
atherosclerotic renal artery disease. Currently, percutaneous intervention with renal artery
stenting is the treatment of choice due to decreased morbidity and mortality risks associated with
surgery.2 Renal artery stenting has been shown to improve blood pressure control and reduce
subsequent episodes of Pulmonary edema in patients with significant RAS. 1 There are also
studies where patients develop further rise in serum creatinine and rapid progression to ESRD
post revascularization. Such deterioration can be explained by renal atheroembolic disease, renal
injury by reactive oxygen metabolites after reperfusion and an imbalance between oxygen
requirement and oxygen delivery leading to renal tissue hypoxia. Patients with severely elevated
creatinine levels, as in the present case generally have a lower likelihood of improvement after
revascularization. However, if left untreated, they face the highest mortality risk but based on
multiple studies intervention offers the potential for significant benefit—If successful, these
patients may regain sufficient renal function to avoid dialysis, making them the group with the
most to gain from treatment.9

Atherosclerotic renovascular disease is rarely sought and many patients face unavoidable fatal
course such as recurrent hospitalizations for heart failure, progression to CKD and chronic
dialysis because they are being treated like any other patients with pulmonary congestion and
renal failure of unknown origin.1 In our case the diagnosis of Pickering syndrome was undetected
for a long time leading to the development of ESRD and dialysis dependence. This case
highlights the importance of maintaining a high index of suspicion for RAS in patients with
recurrent flash pulmonary edema and unexplained renal dysfunction. Advancements in
diagnostic imaging and interventional techniques offer hope for better outcomes, emphasizing
the need for increased awareness and early screening. By identifying and addressing the
underlying cause, the quality of life and long-term prognosis of these patients can be improved.
AUTHOR CONTRIBUTION

Dewasish Swar: Conceptualization, Data curation, Formal analysis, Investigation, Supervision,

Visualization, Writing - original draft, Writing - review & editing

Anju Paudel: Conceptualization, Data curation, Formal analysis, Investigation, Supervision,

Visualization, Writing - original draft, Writing - review & editing

Suman Khanal: Formal analysis, Visualization, Writing - original draft, Writing - review &
editing

Saurav Jha: Data curation, Resources, Writing - original draft, Writing - review & editing

Prabhat K.C: Conceptualization, Formal analysis, Supervision, Validation, Visualization, Writing

- review & editing

SOURCES OF FUNDING

The authors did not receive any funding for this manuscript.

ETHICAL APPROVAL

Not required (from Hospital IRC). In our center, ethical approval is not required for case reports,
only informed written consent from patients is necessary.

CONFLICT OF INTEREST

The authors declare no conflict of interest.

CONSENT

Written informed consent was obtained from the patient’s wife for publication of this case report
and accompanying images in accordance with the journal's patient consent policy.

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FIGURES
Figure 1 Chest Xray showing Pulmonary Edema

Figure 2 Bilateral Renal artery doppler showing bilateral high resistance flow with increased PSV and RI
Figure 3 Right renal angiogram showing critical ostial lesion

Figure 4 Left renal angiogram showing critical ostial lesion